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cystathionine-β-synthetase - translation to English
![Cysteine metabolism. Cystathionine beta synthase catalyzes the upper reaction and [[cystathionine gamma-lyase]] catalyzes the lower reaction.](https://commons.wikimedia.org/wiki/Special:FilePath/Cysteine biosynthesis.svg?width=200 "Cysteine metabolism. Cystathionine beta synthase catalyzes the upper reaction and [[cystathionine gamma-lyase]] catalyzes the lower reaction.")
медицина
цистатионин-β-синтетаза
медицина
пироглутаминовая ацидемия
медицина
гомоцистинурия
Definition
Wikipedia
Cystathionine-β-synthase, also known as CBS, is an enzyme (EC 4.2.1.22) that in humans is encoded by the CBS gene. It catalyzes the first step of the transsulfuration pathway, from homocysteine to cystathionine:
- L-serine + L-homocysteine L-cystathionine + H2O
CBS uses the cofactor pyridoxal-phosphate (PLP) and can be allosterically regulated by effectors such as the ubiquitous cofactor S-adenosyl-L-methionine (adoMet). This enzyme belongs to the family of lyases, to be specific, the hydro-lyases, which cleave carbon-oxygen bonds.
CBS is a multidomain enzyme composed of an N-terminal enzymatic domain and two CBS domains. The CBS gene is the most common locus for mutations associated with homocystinuria.
